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Article*
"Development of the European Network in Orphan Cardiovascular Diseases"
„Rozszerzenie Europejskiej Sieci Współpracy ds Sierocych Chorób Kardiologicznych”
Title: Patient after operation of Tetralogy of Fallot
with pulmonary hypertension
RCD code: Please fill in the code
Author: Lidia Tomkiewicz-Pajak
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital,
Krakow, Poland.
Date: 28.12. 2014
* The article should be written in English]
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
Background, Tetralogy of Fallot is the most common cyanotic congenital heart disease after
one year of age. Less than 5% of all patients with uncorrected ToF survive beyond their 40s.
Prognosis after surgical correction is good with a 32-year survival of 86% [1]. Surgery is
carried out to close ventricular septal defect (VSD) and relieve right ventricular outflow tract
(RVOT) obstruction. Patients who have reached adulthood after earlier repair of Tetralogy of
Fallot frequently present complications. They include severe pulmonary regurgitation (PR),
right ventricular outflow tract obstruction, right ventricular dilation and dysfunction, residual
VSD, aortic root dilation with aortic regurgitation, left ventricular dysfunction, endocarditis,
atrial and ventricular tachycardia, and sudden cardiac death.
Case presentation, A 40-year-old woman after correction of Tetralogy of Fallot was
admitted to hospital due to deterioration in exercise tolerance (NYHA functional class III).
Surgical correction was performed in 1974. Surgical closure of residual VSD was performed
in 2003. A dual chamber pacemaker was implanted due to sick sinus syndrome associated
with paroxysmal atrial fibrillation in 2005. Current treatment included warfarin and
metoprolol CR. Physical examination revealed holosystolic murmur with a thrill at fourth left
intercostal space. Routine laboratory tests were within normal limits, except of elevated
natriuretic peptide level - 1300 pg/ml.
Resting electrocardiogram showed sequential atrial and ventricular pacing. Holter monitoring
didn’t reveal cardiac arrhythmias and pacing disturbances. The cardiopulmonary exercise
testing showed reduced exercise tolerance (workload of 8,4METs) and a reduction in
maximal oxygen consumption - 15,2 ml/kg/min. The observed values were significantly
lower compared previous examinations, February 2011 - 22 ml/kg/min and October 2010 23 ml/kg/min.
The transthoracic echocardiography revealed numerous abnormalities:

enlargement of all heart chambers, impaired RV systolic function, residua
perimembraneous VSD with left to right shunting, severe PR, dilated pulmonary trunk
and branches of pulmonary artery, mild aortic regurgitation, moderate to severe tricuspid
regurgitation,dilated tricuspid annulus, elevated RV systolic pressure (60 mmHg).
Coronary angiography revealed no critical lesions in coronary arteries. Right heart
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
catheterization confirmed presence of residual VSD with left- to- right shunt (ratio of
pulmonary to systemic flow was 2:1). Other findings included mild pulmonary hypertension
(mean pulmonary artery pressure, 29 mmHg) increased pulmonary vascular resistance - 203,9
ARU and increased pulmonary capillary wedge pressure - 16 mmHg.
Discussion, Patients with transannular patch repair of right ventricular outflow tract
obstruction have higher degree of PR compared to patients with subvalvular resection or
repair without a patch [2]. PR may be well tolerated for decades before any symptoms
develop but in some patients can lead to progressive right ventricular dilatation, onset of
tricuspid regurgitation and the need for surgery. It is also known as risk factor for ventricular
arrhythmias and sudden cardiac death [3]. Long-term consequences of PR are augmented by
co-existing distal pulmonary stenosis and pulmonary arterial hypertension. Residual VSD
requiring reintervention after primary surgical repair of Teralogy of Fallot is not rare [4].
Significant left to right shunt through VSD leads to LV volume overload, pulmonary
hypertension should be qualify for surgery. Moreover residual VSD closure, is recommended
if the patient is undergoing pulmonary valve surgery.
Reference. [1] Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients
undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993;329:593–599.
[2] Frigiola A, Redington AN, Cullen S, Vogel M. Pulmonary regurgitation is an important
determinant of right ventricular contractile dysfunction in patients with surgically repaired
tetralogy of Fallot. Circulation. 2004 Sep 14;110(11 Suppl 1):II153-7.
[3] Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac
death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975–981.
[4] Hanna BM, El-Hewala AA, Gruber PJ, et al. Predictive value of intraoperative diagnosis
of residual ventricular septal defects by transesophageal echocardiography. Ann Thorac Surg.
2010 Apr;89(4):1233-7.
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Author’s signature**
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
[** Signing the article will mean an agreement for its publication]
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu